title: [Study of a therapeutic concentrate of factor VIII/vWf prepared in a closed system].
abstract: An original procedure of preparation in a closed system of high purity Factor VIII concentrate is presented. Starting from cryoprecipitates, this method involves a first step of partial removal of fibrinogen by glycine precipitation (1.6 M) and a second step of Factor VIII concentration by cryoprecipitation. The yield is 16.5% of plasmatic F VIII:C (0.8 mu/ml.). Several batches of concentrates thus prepared are compared "in vitro" to 9 other commercially available concentrates from 8 different manufactories. The results show that most of the characteristics of our concentrate are within the range of specifications of other commercially available high-purity F VIII concentrate: F VIII: C activity (CRTS Lille concentrate: 25-40 U/ml.; other concentrates: 25-50 U/ml) solubility, specific activity (CRTS lille concentrate; 1.0-1.82 U F VIII:C/mg protein and 1.79-4.8 U F VIII: C/mg clottable proteins; other concentrates: 0.53-2.79 U F VIII:C/mg protein an 1.39-4.84 U F VIII:C/mg clottable proteins), isoagglutinin titers (CRTS Lille concentrate: 2-8 anti-A, 0.16 anti-B; other concentrates: 0-64 anti-A, 8-16 anti-B) F VIIIC/F VIII R: Ag ratios (CRTS Lille concentrate: 0.18-0.49; other concentrates: 0.20-0.42). Furthermore F VIII R:Ag electrophoretic mobility studied by crossed immunoelectrophoresis add F VIII R: RCo assays provide evidence that very high molecular weight multimeric forms of F VIII/vWf which support vWf activity are present in our concentrate. "In vivo" study and clinical efficacy in vWd patients confirm these results and show that our concentrate is appropriate for the treatment of patients with F VIII:C or V VIII R:RCo deficiency.
mesh_terms: D001777:Blood Coagulation; D001779:Blood Coagulation Factors / Q000302:isolation & purification*; D011232:Chemical Precipitation; D003450:Cryoglobulins; D004587:Electrophoresis, Agar Gel; D005169:Factor VIII / Q000032:analysis / Q000302:isolation & purification* / Q000627:therapeutic use; D005340:Fibrinogen / Q000032:analysis; D006467:Hemophilia A / Q000188:drug therapy; D006801:Humans; D008297:Male; D014842:von Willebrand Diseases / Q000188:drug therapy; D014841:von Willebrand Factor / Q000302:isolation & purification* / Q000627:therapeutic use
chemical_list: D001779:Blood Coagulation Factors; D003450:Cryoglobulins; D014841:von Willebrand Factor; D005169:Factor VIII; D005340:Fibrinogen
vernacular_title: Etude d'un concentré thérapeutique de facteur VIII/vWf préparé en circuit clos.